This is a draft version of the Pathway. The full version will be launched later this year.

Cystic Fibrosis (CF) is one of the most common, life-threatening, recessively inherited diseases in the UK. It is multi-system and progressive, initially affecting mainly the lungs and digestive system, clogging them with thick sticky mucus.

Cystic Fibrosis is the result of mutations affecting a gene that encodes for a chloride channel called the cystic fibrosis transmembrane conductance regulator (CFTR), which is essential for the regulation of salt and water movements across cell membranes. Improper or faulty regulation results in thickened secretions in organs such as the lung and pancreas.

Whilst the pathway is intended as a guide to what should be done at every stage of CF care, it does not dictate how anything should be done. This will always be down the standard practice of the individual Centre or Clinic.

Further Reading:



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